Heterotaxy syndrome is a rare disorder in which children are born with unusual distribution of certain vital organs in the body. This disorder can affect the normal development of certain organs like heart, liver and intestines. Here there is abnormality in the distribution of organs of thoracic and abdominal region. This disorder is evident right from the embryo stage of growth of fetus. For some babies, heart can form on the right side of the body in contrast to the left. Heterotaxy syndrome is also known as situs ambiguous meaning unclear development or unequal distribution of certain organs in the body. However this is a very rare disorder affecting one out of 30,000 births.
Symptoms :
Signs and symptoms vary with respect to the intensity of isomerism and abnormal distribution of organs. Children with Heterotaxy syndrome are likely to develop heart diseases. Mostly they are born with pre-existing heart problem. Cyanotic heart disease develops in children with right isomerism. Some children may develop intestinal problems, malabsorption of nutrients and midgut volvulus later.
Causes :
Exact cause of Heterotaxy syndrome is not known. The disorder becomes evident during the development of fetus which takes wrong position while formation of organs. It can occur possibly due to severe infection or genetic problems or over-exposure of the mother to strong chemicals.
Diagnosis :
Routine ultrasound scanning of the fetus is enough to detect Heterotaxy syndrome. After birth, echocardiogram test and electrocardiogram would be done to identify the intensity of heart problems. The size of the heart can be evaluated through chest X-ray. And for the children with liver or intestinal disorders and positioning, scanning of liver and spleen is to be done.